2446 Idiopathic Non-Cirrhotic Portal Hypertension: A Teaching Case

Abstract

INTRODUCTION: Idiopathic non-cirrhotic portal hypertension (INCPH) is characterized by portal hypertension without cirrhosis in setting of patent hepatic and portal veins, preserved hepatic synthetic function, and no established etiology. We present a case of idiopathic non-cirrhotic portal hypertension. CASE DESCRIPTION/METHODS: A 25-year-old Hispanic man presented with elevated liver enzymes. He was asymptomatic and not taking any medications. His family history was non-contributory. His BMI was 23 and he rarely drank alcohol. His examination was unremarkable. Lab data showed leukopenia to 2700, thrombocytopenia to 96,000, normal INR, AST 80, ALT 114, and alkaline phosphatase (AP) 128. His Alpha 1- Antitrypsin (A1AT), alpha-fetoprotein (AFP), and serum IgG were all normal. The viral hepatitis and HIV serologies were negative. His ANA, ASMA, AMA, anti-LKM titers were also negative. An abdominal sonogram showed liver 14.5 cm and splenomegaly to 14.8 cm; no ascites was seen. A Fibroscan showed F3-F4 consistent with advanced liver fibrosis, but our liver biopsy had <5% fatty change, negative iron stain, and overall normal histology. This was confirmed by a second outside pathologist. Testing for other diagnoses including Budd-Chiari, Epstein-Barr virus, cytomegalovirus, and Schistosomiasis returned negative. DISCUSSION: Non-cirrhotic portal hypertension can present with lab, imaging, and endoscopic findings suggestive of cirrhosis, yet liver biopsy shows normal histology. Our patient underwent extensive workup for cirrhosis and causes of non-cirrhotic portal hypertension before we diagnosed him with INCPH. This is a pre-sinusoidal entity that involves: 1) presence of portal hypertension, 2) absence of cirrhosis or advanced liver disease, and 3) lack of hepatic or portal vein thrombosis. Unlike in cirrhosis, liver failure, ascites, and hepatic encephalopathy are not typically present. Patients have preserved liver function and often normal LFTs, but can have splenomegaly (95%), hepatomegaly (50%), varices, and thrombocytopenia. The most common complication in these patients is variceal bleeding. Thus, primary and secondary prevention of esophageal bleeding via non-selective beta blockers and endoscopic therapy is beneficial. Of note, there is no role for screening patients for hepatocellular carcinoma (HCC) in INCPH since the absence of cirrhosis renders the HCC risk very low. INCPH is important to consider in patients with no clear cause for portal hypertension or LFT abnormalities.